The pathological picture in the Swedish family was reminiscent of PSP also in aspects other than 4R predominance: (1) the presence of neuronal NFTs and, albeit scarce, of tufted astrocytes and occasional coiled bodies [22, 23]; (2) prominent tau white matter pathology, and (3) marked involvement of the brainstem and central nuclei. The gene discussed is MAPT; the disease is supranuclear palsy, progressive, 1.